Publication
Title
The transition from normal lung anatomy to minimal and established fibrosis in idiopathic pulmonary fibrosis (IPF)
Author
Abstract
Background The transition from normal lung anatomy to minimal and established fibrosis is an important feature of the pathology of idiopathic pulmonary fibrosis (IPF). The purpose of this report is to examine the molecular and cellular mechanisms associated with this transition. Methods Pre-operative thoracic Multidetector Computed Tomography (MDCT) scans of patients with severe IPF (n = 9) were used to identify regions of minimal(n = 27) and established fibrosis(n = 27). MDCT, Micro-CT, quantitative histology, and next-generation sequencing were used to compare 24 samples from donor controls (n = 4) to minimal and established fibrosis samples. Findings The present results extended earlier reports about the transition from normal lung anatomy to minimal and established fibrosis by showing that there are activations of TGFBI, T cell co-stimulatory genes, and the down-regulation of inhibitory immune-checkpoint genes compared to controls. The expression patterns of these genes indicated activation of a field immune response, which is further supported by the increased infiltration of inflammatory immune cells dominated by lymphocytes that are capable of forming lymphoid follicles. Moreover, fibrosis pathways, mucin secretion, surfactant, TLRs, and cytokine storm-related genes also participate in the transitions from normal lung anatomy to minimal and established fibrosis. Interpretation The transition from normal lung anatomy to minimal and established fibrosis is associated with genes that are involved in the tissue repair processes, the activation of immune responses as well as the increased infiltration of CD4, CD8, B cell lymphocytes, and macrophages. These molecular and cellular events correlate with the development of structural abnormality of IPF and probably contribute to its pathogenesis.
Language
English
Source (journal)
EBioMedicine
Publication
2021
ISSN
2352-3964
DOI
10.1016/J.EBIOM.2021.103325
Volume/pages
66 (2021) , p. 1-10
Article Reference
103325
ISI
000646005100004
Pubmed ID
33862585
Full text (Publisher's DOI)
Full text (open access)
UAntwerpen
Research group
Publication type
Subject
External links
Web of Science
Record
Identifier
Creation 25.01.2024
Last edited 26.01.2024
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